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Vasculitic Neuropathy

  • David A. Chad
  • Peter Siao

Abstract

Vasculitic neuropathy results from inflammation within the blood vessel walls of the vasa nervorum, the blood vessels that supply peripheral nerves. The forms of systemic vasculitis most likely to be associated with vasculitic neuropathy are polyarteritis nodosa, microscopic polyangiitis, the Churg—Strauss syndrome, Wegener's granulomatosis, and mixed cryoglobulinemia. Large-vessel vasculitides such as giant cell arteritis and Takayasu's arteritis seldom cause vasculitic neuropathy. Similarly, vasculitic neuropathy is seldom (if ever) a complication of the types of vasculitis that involve the smallest blood vessels, e.g., cutaneous leu-kocytoclastic angiitis (hypersensitivity vasculitis) and Henoch—Schönlein purpura. Rather, vasculitic neuropathy tends to be caused by systemic vasculitides that have the ability to target medium-sized blood vessels. Vasculitic neuropathy can also occur as a disease process that is limited to the peripheral nerves, sparing other organs. Such cases are sometimes referred to as “isolated vasculitis of the peripheral nervous system” or “nonsystemic vasculitic neuropathy.” Vasculitic neuropathy is generally a “length-dependent” disease process. Thus, the distal extremities tend to be involved more extensively than are the proximal extremities and trunk importantly, vasculitic neuropathy can also present initally as a multifocal, nonlength dependent process (arms affected earlier than legs). Vasculitic neuropathy has the potential to cause serious morbidity (persisting weakness) through the resultant infarctions of peripheral nerves. The combination of cyclophosphamide and high doses of glucocorticoids is an appropriate treatment regimen for most patients with vasculitic neuropathy.

Keywords

Giant Cell Arteritis Nerve Conduction Study Systemic Vasculitis Nerve Biopsy Mixed Cryoglobulinemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Bradley WG, Chad D, Verghese JP, Liu HC, Good P, Gabbai AA, Adelman LS. Painful lumbosacral plexopathy with elevated eryth-rocyte sedimentation rate: a treatable inflammatory syndrome. Ann Neurol. 1984;15:457–64PubMedCrossRefGoogle Scholar
  2. Burns TM, Schaublin GA, Dyck PJ. Vasculitic neuropathies. Neurol Clin. 2007;25:89–113PubMedCrossRefGoogle Scholar
  3. Collins MP, Periquet MI. Non-systemic vasculitic neuropathy. Curr Opin Neurol. 2004;17:587–98PubMedCrossRefGoogle Scholar
  4. David WS, Peine C, Schlesinger P, Smith SA. Nonsystemic vasculitic mononeuropathy multiplex, cryoglobulinemia, and hepatitis C. Muscle Nerve. 1996;19:1596–602PubMedCrossRefGoogle Scholar
  5. Dyck PJ, Benstead TJ, Conn DL, et al Nonsystemic vasculitic neuropathy. Brain 1987;110:843–54PubMedCrossRefGoogle Scholar
  6. Dyck PJ, Conn DL, Okazaki H. Necrotizing angiopathic neuropathy: three dimensional morphology of fiber degeneration related to sites of occluded vessels. Mayo Clin Proc. 1972;47:461–75PubMedGoogle Scholar
  7. Kissel JT, Mendell JR. Vasculitic neuropathy. Neurol Clin. 1992;10: 761–81PubMedGoogle Scholar
  8. Lacomis D, Giuliani MJ, Steen V, et al Small fiber neuropathy and vas-culitis. Arthritis Rheum. 1997;40:1173–7PubMedCrossRefGoogle Scholar
  9. Lacomis D, Zivkovic SA. Approach to vasculitic neuropathies. J Clin Neuromusc Dis. 2007;9:265–76CrossRefGoogle Scholar
  10. Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinat-ing neuropathy with persistent conduction block. Neurology 1982; 32:958–64PubMedCrossRefGoogle Scholar
  11. Mathew L, Talbot K, Love S, et al Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. QJM. 2007; 100:41PubMedCrossRefGoogle Scholar
  12. Oh SJ. Paraneoplastic vasculitis of the peripheral nervous system. Neurol Clin. 1997;15:849–63PubMedCrossRefGoogle Scholar
  13. Pestronk A. Multifocal motor neuropathy: diagnosis and treatment. Neurology 1998;51:S22–24PubMedCrossRefGoogle Scholar
  14. Said G, Lacroix-Ciaudo C, Fujimura H, Blas C, Faux N. The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol. 1988;23:461–5PubMedCrossRefGoogle Scholar
  15. Said G. Necrotizing peripheral nerve vasculitis. Neurol Clin. 1997; 15: 835–48PubMedCrossRefGoogle Scholar
  16. Seo J-H, Ryan HF, Claussen GC, Thomas TD, Oh SJ. Sensory neuropathy in vasculitis: a clinical, pathological, and electrophysiologic study. Neurology 2004;63:874–8PubMedCrossRefGoogle Scholar
  17. Schaumburg HH, Berger AR, Thomas PK. Disorders of peripheral nerves (Contemporary Neurology Series 36). 2nd ed. Philadelphia: FA Davis; 1992. p. 130–5Google Scholar
  18. Vallat J-M, Cros DP, Hedley-White ET. Case 9-2007: a 27 year-old woman with pain and swelling of the legs. N Engl J Med. 2007; 356:1252–9PubMedCrossRefGoogle Scholar
  19. Vital C, Vital A, Canron MH, Jaffre A, Viallard JF, Ragnaud JM, Brechenmacher C, Lagueny A. Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases. J Peripher Nerv Syst. 2006;11:20–9PubMedCrossRefGoogle Scholar
  20. Vrancken AF, Hughes RA, Said G, et al Immunosuppressive treatment for non-systemic vasculitic neuropathy. Cochrane Database Syst Rev. 2007;24(1):CD006050Google Scholar
  21. Wees SJ, Sunwoo IN, Oh SJ. Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med. 1981;71:525–32PubMedCrossRefGoogle Scholar
  22. Živković SA, Ascherman D, Lacomis D. Vasculitic neuropathy: elec-trodiagnostic findings and association with malignancies. Acta Neurol Scand. 2007:115;432–6PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • David A. Chad
    • 1
  • Peter Siao
    • 1
  1. 1.Neurology DepartmentMassachusetts General HospitalBostonUSA

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